Kimura's disease in children: A 9 years prospective study

SummaryKimura's disease is a rare form of chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region and frequently associated with lymphadenopathy and/or salivary gland enlargement. The nodular lesions are deep seated in subcutaneous tissue and clinically may mimic a neoplasm. Hence head and neck surgeons need to be aware of clinical presentation of Kimura's disease.ObjectivesTo study the clinical presentations, management and complications of Kimura's disease in pediatric age group.Study designProspective study.Materials and methodsThe duration of study was 9 years (January 1998 to December 2006), comprising of 18 patients. Only histopathologically proven cases were included in this study. Blood eosinophil count and serum IgE estimation were done in all these patients. All the patients underwent fine needle aspiration cytology study. In 15 patients excision biopsy was done and resected specimen was sent for histopathological examination. In three cases, only biopsy was done to confirm the diagnosis.ResultsAll the patients presented with painless swelling in the head and neck region. Post-auricular region was the commonest site (50%). Sixteen patients (88.8%) had blood eosinophilia and in 15 patients (83.3%) serum IgE level was elevated. Fifteen cases were treated by surgery and three cases were treated with steroids. Out of 18 cases, 15 cases were symptom free at the end of 1 year. Among 15 patients who underwent surgery, only one had recurrence (6.6 %). Out of three patients who were treated with corticosteroids, two came back with recurrence (66.6%). In our study, totally three patients had recurrence (16.6%). One patient had nephrotic syndrome (5.5%).ConclusionPost-auricular region is the commonest site of involvement. Incidence is more common in the second decade of life. Recurrence rate is more with steroid therapy. Surgery is the best modality of treatment. The only systemic complication is nephrotic syndrome.